POS0890 NINTEDANIB REAL-LIFE EFFICACY AND SAFETY IN SYSTEMIC SCLEROSIS (SSc)-INTERTISTIAL LUNG DISEASE (ILD): AN ITALIAN MULTICENTRE PRELIMINARY STUDY.
Authors: C. Campochiaro, G. De Luca, M. G. Lazzaroni, G. Armentaro, A. Spinella, B. Vigone, B. Ruaro, A. Stanziola, D. Benfaremo, E. De Lorenzis, F. Benvenuti, S. L. Bosello, G. Moroncini, G. Cuomo, M. Confalonieri, L. Beretta, E. Zanatta, D. Giuggioli, N. Del Papa, P. Airò, L. Dagna, M. Matucci-Cerinic
Journal: Annals of the Rheumatic Diseases (2022)

Background Nintedanib (NTD) has been approved for Systemic Sclerosis (SSc)-Interstitial Lung Disease (ILD) following the positive results of the SENSCIS trial. Objectives to describe the efficacy and safety of NTD in SSc-ILD in a real-life setting. Methods The clinical data of SSc-ILD patients treated with NTD from 10 Italian SSc centres were retrospectively evaluated at baseline, 6 and 12 months: SSc clinical features, NTD tolerability, pulmonary function tests (PFTs) and modified Rodnan skin score (mRSS) were recorded. Results 69 SSc-ILD patients (22 males [32%], mean age 60±12 years, disease onset 50±13 years, 4 [6%] anti-centromere, 53 [77%] anti-topoisomerase I, 3 [4%] anti-RNA-polimerase III) were identified. The vast majority (84%) was previously treated with immunosuppressants: 27 (39%) cyclophosphamide, 45 (65%) mycophenolate mofetil, 6 (9%) methotrexate, 9 (13%) azathioprine, 6 (9%) tocilizumab and 22 (32%) rituximab. In 11 (16%) patients, NTD was the first treatment for SSc-ILD. At baseline, 57 patients (83%) were on corticosteroids (mean daily prednisone dose 6±5 mg), 58 (84%) on immunosuppressants, 47 (68%) on mycophenolate mofetil, 14 (20%) on rituximab, 3 (4%) on tocilizumab, 2 on methotrexate (3%) and 1 (1%) on azathioprine. At baseline HRCT showed UIP pattern in 27 (39%) and NSIP pattern in 42 (61%) patients. The modifications of PFTs and mRSS over time are shown in Table 1. Since NTD introduction, gastro-intestinal (GI) side effects were recorded in 34 (49%) patients, with diarrhoea being the most common complaint (35%), followed by nausea/vomiting (23%) and weight loss (16%). In 21 (30%) patients, after a mean time of 2.6±3.4 months, NTD was maintained after dose adjustment. In 5 (7%) patients NTD was stopped after a median time of 5 (1-6) months due to subocclusion and persistent diarrhoea in 3 patients, untreatable nausea and vomiting in one patient and liver toxicity in 1 patient. During the follow-up after a median time of 10 (6 – 33) months, 4 patients died. Table 1. Pulmonary function tests and mRSS at baseline, 6 and 12 months in SSc-ILD on NTD. Baseline 6 months P value Baseline 12 months P value FVC (% predicted) 64 ± 18 65 ± 18 (33 pts) 0.638 70 ± 19 69 ± 18 (20 pts) 0.586 TLC (% predicted) 64 ± 15 61 ± 14 (27 pts) 0.154 64 ± 14 65 ± 18 (16 pts) 0.944 DLCO (% predicted) 40 ± 17 41 ± 18 (29 pts) 0.660 40 ± 18 38 ± 18 (20 pts) 0.304 mRSS 9 ± 6 8 ± 6 (26 pts) 0.002 7 ± 4 8 ± 6 (15 pts) 0.334 pts= patients Conclusion Our preliminary data confirm that in a real-life clinical scenario NTD, in combination with immunosuppressants, may stabilize PFT. However, despite the fact that GI side effects are frequent, they may be controlled with NTD dose adjustment thus retaining the drug in SSc-ILD patients. The NTD efficacy on skin involvement needs to be thoroughly evaluated on a larger SSc population. Disclosure of Interests Corrado Campochiaro Speakers bureau: Boeboehringer ingelheim, Giacomo De Luca Speakers bureau: boehringer ingelheim, Maria Grazia Lazzaroni Grant/research support from: boehringer ingelheim, Giuseppe Armentaro: None declared, Amelia Spinella: None declared, Barbara Vigone: None declared, Barbara Ruaro: None declared, Anna Stanziola: None declared, Devis Benfaremo: None declared, Enrico De Lorenzis: None declared, Francesco Benvenuti: None declared, Silvia Laura Bosello Speakers bureau: boehringer ingelheim, Gianluca Moroncini: None declared, Giovanna Cuomo: None declared, Marco Confalonieri: None declared, Lorenzo Beretta: None declared, Elisabetta Zanatta: None declared, Dilia Giuggioli: None declared, Nicoletta Del Papa: None declared, Paolo Airò: None declared, Lorenzo Dagna: None declared, Marco Matucci-Cerinic Speakers bureau: boehringer ingelheim